Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a very rare congenital disease characterized by abdominal distension due to a dilated urinary bladder, microcolon and decreased or absent intestinal peristalsis. Most patients die at an early age. We report on a female baby with this syndrome which was associated with duodenal web and tracheobronchial malacia. The literature on this syndrome is also reviewed.